We know little about the ability to explore and navigate large-scale space for people with intellectual disabilities (ID). In this cross-syndrome study, individuals with Down syndrome (DS), individuals with Williams syndrome (WS) and typically developing children (TD; aged 5 to 11 years) explored virtual environments with the goal of learning where everything was within the environment (Experiment 1) or to find six stars (Experiment 2). There was little difference between the WS and DS groups when the goal was simply to learn about the environment with no specific destination to be reached (Experiment 1); both groups performed at a level akin to a subset of TD children of a similar level of non-verbal ability. The difference became evident when the goal of the task was to locate targets in the environment (Experiment 2). The DS group showed the weakest performance, performing at or below the level of a subset of TD children at a similar level of non-verbal ability, whilst the WS group performed at the level of the TD subset group. The DS, WS and TD group also demonstrated different patterns of exploration behavior. Exploration behaviour in DS was weak and did not improve across trials. In WS, exploration behavior changed across trials but was atypical (the number of revisits increased with repeated trials). Moreover, transdiagnostic individual difference analysis (Latent Profile Analysis) revealed five profiles of exploration and navigation variables, none of which were uniquely specific to DS or to WS. Only the most extreme profile of very poor navigators was specific to participants with DS and WS. Interestingly, all other profiles contained at least one individual with DS and at least one individual with WS. This highlights the importance of investigating heterogeneity in the performance of individuals with intellectual disability and the usefulness of a data-driven transdiagnostic approach to identifying behavioral profiles.
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Individuals with Down Syndrome (DS) and individuals with Williams syndrome (WS) present with poor navigation and elevated anxiety. The aim of this study was to determine the relationship between these two characteristics. Parent report questionnaires measured navigation abilities and anxiety in WS (N = 55) and DS (N = 42) as follows. Anxiety: Spence Children's Anxiety Scale and a novel measure of navigation anxiety. Navigation: Santa Barbara Sense of Direction Scale (SBSOD) and a novel measure of navigation competence. Most individuals were not permitted to travel independently. A relationship between navigation anxiety and SBSOD scores (but not navigation competence) was observed for both groups.
Abstract This chapter shows that executive functions and motor planning are impaired in Williams syndrome (WS). It assesses inhibition (such as inhibition of a prepotent response) and its influence on social functioning, and evaluates how the cascading developmental effects of poor planning ability impact on the wider functioning of other aspects of cognition such as performance on visuospatial tasks. It considers studies that have investigated the interplay between multiple executive functions to resolve the mixed evidence relating to executive dysfunction in WS. Motoric planning is discussed in terms of walking, stair-descent, and reaching studies that reveal evidence for poor motor planning in WS. The chapter highlights how the use of neuroimaging techniques and analysis of movement in real-world situations has advanced the understanding of executive function and motor planning in typical development as well as in groups with neurodevelopmental disorders including WS.